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中文摘要
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为了评估生殖健康,我们联系了39名患有纤维发育不良/麦考恩-奥尔布赖特综合征的妇女,并获得了详细的月经和生育史。此外,还回顾了卵巢超声结果以评估自发性囊肿的存在,并回顾了生化数据。 我们发现77%(30/39)的妇女报告异常子宫出血,导致严重贫血需要输血的3例。9名女性接受了子宫切除术以控制出血,其中67%(6/9)的患者年龄不到35岁,非常年轻。43%的妇女(9/21)受累于不孕症,其中2名妇女在接受卵巢囊肿切除术后发展为原发性卵巢功能不全。在14名妇女的25次自然怀孕中,35%(8)是计划外怀孕。在14个受试者中,有7个受试者的骨痛没有变化(53%),4个受试者的骨痛增加(31%),2个受试者的骨痛减轻(15%)。在怀孕期间没有其他骨骼并发症的报告。 我们的结论是,患有纤维发育不良/麦考恩-奥尔布赖特综合征的女性报告妇科发病率高,生育力降低。在这一人群中,怀孕和不良的骨骼结局之间没有明显的联系。 在这项先导性、双盲、安慰剂对照试验中,患有产后抑郁的妇女被随机分成两组,分别接受17-雌二醇透皮贴片(100微克/天)或安慰剂贴片。在超过6周的时间里,女性完成了贝克抑郁量表(BDI)、爱丁堡产后抑郁量表(EPDS)和汉密尔顿抑郁量表(HAM-D)的每周评分。主要结果测量为6周时的治疗反应(较基线BDI下降50%)和缓解(BDI<10),次要结果测量包括第3周和第6周所有量表的严重程度。在12名招募的女性中,6人接受TE,6人接受安慰剂治疗。到第6周,接受TE治疗的5名妇女和4名症状缓解,相比之下,安慰剂组有2名应答者和1名缓解者。差异无统计学意义(p=0.24)。在BDI分级的混合模型中,与安慰剂相比,TE在3周时与安慰剂相比下降了9.2点(95%可信区间-19.5点至+1.0点,p=0.074),在6周时下降10.5点(95%可信区间-21.0-0.0点,p=0.049),尽管这些差异不能通过多次比较校正。对HAM-D评分有类似的影响,但对EPDS评分没有。有趣的是,不同组间的血浆雌二醇水平没有显著差异。与安慰剂相比,我们不能证明TE对产后抑郁有显著的治疗益处。尽管受到招募不足和缺乏随访的限制,我们的结果表明TE是门诊PPD管理的一个可行的选择,有初步证据(基于二次结果)证明疗效。治疗效果最早可在3周内显现,可能不直接依赖于外周测量的雌二醇量。我们推测,与脉动的内源性雌激素相比,TE可能提供更一致的雌激素暴露,因为分娩后正常的垂体-卵巢轴恢复,而更一致的雌激素时间可能是接受TE的女性改善的基础。
英文摘要
To evaluate reproductive health, we contacted thirty-nine women with fibrous dysplasia/McCune-Albright syndrome and obtained detailed menstrual and reproductive histories. In addition, ovarian ultrasound results were reviewed to evaluate for the presence of a spontaneous cyst, and biochemical data were reviewed. We found that 77% (30/39) of the women reported abnormal uterine bleeding that caused severe anemia requiring blood transfusion in 3 cases. Nine women underwent hysterectomy for management of bleeding, including 67% (6/9) at the unusually young age of less than age 35years. Infertility affected 43% of women (9/21), including 2 women who developed primary ovarian insufficiency after undergoing surgical resection of ovarian cysts. Of 25 spontaneous pregnancies in 14 women, 35% (8) were unplanned. Among the 14 pregnancies, pregnancy was associated with no change in bone pain in 7 subjects (53%), increased bone pain in 4 subjects (31%), and decreased bone pain in 2 subjects (15%). No additional skeletal complications were reported during pregnancies. We conclude that women with fibrous dysplasia/McCune-Albright syndrome report a high prevalence of gynecologic morbidity and reduced fertility. There is no clear association between pregnancy and poor skeletal outcomes in this population. In this pilot, double-blind, placebo-controlled trial, women with PPD were randomized to receive transdermal 17-estradiol (100 mcg/day) or placebo patch. Over 6 weeks, women completed weekly ratings on the Beck Depression Inventory (BDI), Edinburgh Postnatal Depression Scale (EPDS), and Hamilton Depression Scale (HAM-D). Primary outcome measures were treatment response (>50% decrease from baseline BDI) and remission (BDI <10) at 6 weeks, and secondary outcome measures included severity on all scales at weeks 3 and 6. Of 12 recruited women, 6 received TE and 6 received placebo. By week 6, 5 women receiving TE responded to treatment and 4 showed symptom remission, compared to 2 responders and 1 remitter in the placebo group. This difference was not significant (p=0.24). In a mixed-model of BDI ratings, TE was associated with a 9.2 point decrease at 3 weeks (95%CI -19.5 to +1.0, p=0.074) and a 10.5 point decrease at 6 weeks (95%CI -21.0-0.0, p=0.049) compared to placebo, though these differences did not survive multiple comparisons correction. Analogous effects were found for HAM-D but not EPDS scores. Interestingly, no significant difference in plasma estradiol levels existed between groups. We were unable to demonstrate a significant therapeutic benefit of TE compared with placebo in PPD. Although limited by under-recruitment and loss to follow-up, our results suggest TE is a feasible option for outpatient PPD management, with preliminary evidence (based on secondary outcomes) for efficacy. Therapeutic effects may be seen as early as 3 weeks and may not directly depend on peripheral measures of estradiol. We speculate that TE may provide more consistent estrogen exposure than pulsatile endogenous estrogen as the normal pituitary-ovarian axis resumes after delivery, and that a more consistent estrogenic mileau may underlie the improvements in the women who received TE.
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